Friedreich’s Ataxia (FRDA) is the most common inherited human ataxia and is caused by a deficiency in the mitochondrial protein frataxin. Clinically, patients suffer progressive spinocerebellar degeneration, diabetes, and a fatal cardiomyopathy, associated with mitochondrial respiratory defects. Recent findings have shown that lysine acetylation regulates mitochondrial function and intermediary metabolism. However, little is known about lysine acetylation in the setting of pathological energy stress and mitochondrial dysfunction.